Missed Doses of Kalydeco and Standard CF Protocol
My last entry described what happened when Brady caught a stomach virus that led him to miss several days of Kalydeco. He developed a cough as the stomach symptoms subsided, so we started him back on HyperSal treatments to help him clear junk out of his lungs (We had cut out daily HyperSal treatments months ago, as he no longer needed them for maintenance). The cough quickly worsened. It was a barky sounding "bronchospastic" cough that Albuterol wouldn't calm a bit. After a few days of this, Brady's Dr. prescribed Augmentin and a burst of Prednisone to ease the inflammation in his lungs. The Prednisone helped immensely, but the cough didn't completely go away. We stopped HyperSal for a few days (around the time I wrote my last entry), but restarted only a few days later after hearing a couple of suspicious coughs.
Once again, Brady's cough took a sharp turn for the worse and sounded exactly as before--bronchospasms. I couldn't understand what was causing this. Had he acquired a serious lung infection that Augmentin hadn't touched? Brady has had years of clean cultures...and has a CT scan showing NO VISIBLE LUNG DAMAGE...and a sweat test score well below the diagnostic level for CF. What the hell?! I had a sick feeling in my stomach and was so confused about what was going on. This time, Brady's Dr. requested a new throat culture, and started him on another short burst of Prednisone for the bronchospasms.
As we waited for the throat culture results, I wracked my brain for answers. I was a mess. In my mind, I had convinced myself that if we could get Kalydeco to Brady while his lungs were still very healthy, that we might be able to spare him a lifetime of fighting lung infections. Had I been terribly wrong? Would he culture Pseudomonas or some other CF specific lung infection for the first time ever AFTER being on Kalydeco for 15 months?!
The cough improved again with Prednisone, but didn't go completely away. Brady's nurse called with the new culture results--NORMAL FLORA. We were relieved, but still full of questions. His Dr. wasn't sure what to do, but was anxious to see Brady return to baseline. His recommendation was to start him on a different antibiotic for the cough. Something wasn't adding up for me. I didn't feel like the Augmentin had helped at all and I didn't think a new antibiotic was the answer either. I didn't believe he had an infection at all! Had he developed serious asthma? Some kind of new allergy?
I called my parents to share Brady's throat culture report. Looking for answers, we retraced in detail the course Brady's cough had taken, and then it happened--I had an epiphany. It hit me like a flash, and a punch in the gut at the same time. I knew what was causing Brady's cough...and it wasn't an infection, asthma, or an allergy. WE WERE CAUSING BRADY'S COUGH WITH THE HYPERSAL TREATMENTS!
As soon as I realized it, everything made sense. Brady started HyperSal when he was 20 months old, and tolerated twice a day treatments with absolutely no problem and very little coughing... until he started Kalydeco. We noticed his tolerance to HyperSal change quite a bit right after he began Kalydeco. The treatment suddenly seemed a lot more irritating and produced more coughing than before it ever had before. Because Kalydeco theoretically does the job of thinning mucous on it's own, we decided that HyperSal would be the first treatment to go. It has been months since he has taken HyperSal, but I never dreamed his reaction to the treatment would be so dramatically different. Before Kalydeco, we felt like Brady really NEEDED the HyperSal, and we considered it one of our greatest tools in fighting away CF bugs and coughs. Had things really changed so much?
I thought back to Brady's first ever HyperSal treatment as a baby. His Dr. made us trial it in the clinic, because it can be very irritating...and for some patients, can cause bronchospasms. I remembered that patients in the clinical trials for VX-770 were not allowed to use HyperSal, but I had never heard an explanation for why it was excluded. Yup, it all was all starting to make sense. As this swirling realization came together in my head, my epiphany was quickly followed by nausea.
What had we done? Poor Brady. The more he had coughed, the more HyperSal we gave him...because that is how you treat a CF cough. We gave him antibiotics and Prednisone...because that is what you do to make a CF cough go away. I was crushed thinking of all that time Brady spent coughing and all those drugs he swallowed for NO REASON! Be gentle with me on the comments please, because trust me, I feel so bad for not realizing sooner and putting him through all that.
Standard CF protocol isn't working for Brady anymore. His biology is drastically different now. This HyperSal incident was just the brutal slap in the face I needed to see that I can't rely on the same old line of thinking. Numerous tests have shown us that Brady's body is working "normally." In the CF world, doing treatments is how we protect and care for our children; it was so hard to grasp that my good intentions had gone so wrong.
Now I see
I realized that my overprotective instincts regarding Brady's health are not only unnecessary--they could be dangerous. I realized that Brady didn't need a new antibiotic or steroids or MORE treatments to get well, he needed to be left alone to let his body heal on its own. I realized that by stepping in and pumping up breathing treatments at the first sign of a cough...we had PROVOKED a health crisis in my sweet boy. I realized the answer was to stop treating him so much like he has CF and start treating him more like a regular healthy kid...because he is. Jesus. I drank wine and cried for a week after my epiphany.
I called the CF clinic and explained my theory to them.
Brady's Pulmonologist said it was a "fascinating theory," and gave us a few days to test it out before we went ahead with any more antibiotics. Once we stopped HyperSal, the cough started to get better right away. Now, Brady is totally back to normal and all is right with the world again. Not only was Kalydeco working like it should...it was working better than my tiny little brain could even IMAGINE! Since my epiphany, I've thought a lot more about other ways we might be "overmedicating" Brady, now that he is taking Kalydeco. CF clinic is coming up this Thursday and I am anxious to talk to his Doc. We are going to discuss all of Brady's drugs, whether his condition merits continued use, and come up with a systematic plan to bring him off the unneeded therapies, one-at-a-time. I'm not going to just FREAK OUT and drop anything that he needs, but as we've seen, overmedicating can also have negative consequences. I am certainly not suggesting that everyone taking Kalydeco stop doing any of their other treatments. I understand that those who have suffered significant lung damage may still need many of their drugs and therapies to stay healthy, but Brady was only 4 1/2 when he started this game-changing drug and his case may be different. I know all CF parents can relate when I say how extremely difficult it can be to make healthcare decisions for your child. Now I understand that I might have to venture outside my "CF box" to do the right thing for Brady.